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The Dutch Idiopathic Ventricular Fibrillation Registry is one of the largest national cohorts with patients initially diagnosed with idiopathic ventricular fibrillation.
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During follow-up, 9% received a diagnosis other than ‘idiopathic’ and 25% received appropriate implantable cardioverter defibrillator therapy.
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Non-invasive electrocardiographic imaging and other diagnostic modalities are used to reveal underlying substrates.
Introduction
Methods
Objectives
Study population
Data collection
Electrocardiographic imaging
Results
All | Idiopathic | Diagnosis | |
---|---|---|---|
Total | 432 (100%) | 394 (91%) | 38 (9%) |
Age at index event (years)a | 40 (28–52) | 40 (29–52) | 38 (26–54) |
Male sex | 262 (61%) | 237 (60%) | 25 (66%) |
Family history of SCD | 67/424 (16%) | 59/388 (15%) | 8/36 (22%) |
Follow-up duration (years)a | 6 (2–12) | 6 (2–12) | 11 (6–17) |
Follow-up available > 1 year | 345 (80%) | 310 (79%) | 35 (92%) |
Diagnostic work-upa | |
---|---|
Electrocardiogram | 432 (100%) |
Toxicological screening | 66 (15%) |
Holter telemetry | 281 (65%) |
Echocardiography | 423 (98%) |
Exercise treadmill test | 306 (71%) |
Coronary imaging | 404 (94%) |
Cardiac magnetic resonance | 323 (75%) |
Sodium channel blocker provocation | 274 (63%) |
Ergonovine provocation | 80 (19%) |
Genetic testing | 376 (87%) |
All high-yield diagnostic tests performed | 175 (41%) |
Clinical course
Diagnosis
Diagnosis | Number of patients | Findings before diagnosis |
---|---|---|
Total | 38 (100%) | |
Cardiomyopathy | 18 (47%) | |
– Arrhythmogenic cardiomyopathy | 9 (24%) | 9 patients with non-diagnostic findings at baseline (100%) |
– Dilated cardiomyopathy | 5 (13%) | 2 patients with non-diagnostic findings at baseline (40%) |
– Hypertrophic cardiomyopathy | 4 (10%) | 4 patients with non-diagnostic findings at baseline (100%) |
Channelopathy | 14 (37%) | |
– Brugada syndrome | 5 (13%) | 3 patients without provocation test at baseline, resulting in a diagnosis during follow-up (60%) |
– Long-QT syndrome | 1 (3%) | |
– Catecholaminergic—polymorphic VT | 5 (13%) | 5 patients with ectopy during diagnostic testing at baseline (100%) |
– Early repolarisation syndrome | 3 (8%) | |
Myocarditis | 1 (3%) | |
Coronary artery spasm | 2 (5%) | |
Other | 3 (8%) |